Download Usher Screening

CD and Vision Screening Instrument for Children who are
Deaf or Hard of Hearing
Susan R. Easterbrooks
Dept of Educational Psychology and Special Education
Georgia State University
Debbie Parkman
Georgia Deafblind Project
Georgia State University
Paper Presented at the 27th Annual Conference of the Association of College
Educators- Deaf/Hard of Hearing. San Diego, CA, February 23, 2001
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Vision Screening Instrument for Children who are Deaf or Hard of Hearing
The purpose of this presentation is to demonstrate the use of a newly developed
screening devise for use in identifying vision problems in students who are deaf or hard
of hearing. In order to make this tool more accessible to teachers of the deaf, an
instructional CD was created that describes the screening kit. In this presentation we
show the kit and the CD to the audience.
Background Information on Vision Screening
What Exactly Is the Vision Screening Instrument for Children who are Deaf of Hard of
Hearing?
This kit was designed by members of the Georgia Deafblind project for the
purpose of providing classroom teachers, parents and school nurses with a tool for
assessing vision loss in the deaf and hard of hearing population. According to
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% of students with hearing losses have vision problems, and many of these remain
undetected throughout the child’s school years. This is a tragic situation since so many
students with hearing losses depend on vision as their primary source of information. The
kit was developed as a referral instrument. It is not a diagnostic tool. Only
ophthalmologists and optometrists are trained and licensed to perform diagnostic testing.
This tool is designed so that teacher, parents and school nurses may identify
characteristics and behaviors that would suggest the need for a child to be referred to an
ophthalmologist or optometrist.
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What is the Purpose of the Screening Tool
This screening tool has two purposes: 1) to screen for vision loss in children who
are deaf or hard of hearing, and 2) to provide schools with a comprehensive assessment
that screens for specific characteristics associated with Usher Syndrome. Usher
Syndrome is described in depth later in this paper.
A vision screening is recommended for children who are six years of age or older.
A screening should be repeated at least every two years because vision status changes
over time. Some portions of this screening may require two people to administer them.
Who Gets Screened?
Any child who has been diagnosed with a hearing loss should receive a vision
screening. It does not matter whether that child has a mild or a profound loss. All children
with hearing losses, and all children for that matter, use their sense of vision as an
important instructional tool, so any effort to enhance this sense will improve a student’s
chances of learning better. This tool can be used both in day or residential programs for
the deaf and in regular education programs serving children with hearing losses. It should
be used no matter what the communication philosophy of a school, whether they support
oral instruction, the use of English-based signs or the use of American Sign Language
(ASL).
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Who Does the Screening?
Just about any responsible adult who has received training in the administration of
this device can do the screening; however, the tool was developed with three groups in
mind: teachers of the deaf and hard of hearing, 2) teachers of the visually impaired, and
3) school nurses. Administrators might also want to provide training to parents and to
other educational diagnosticians in their facility.
How Can I Get a Screening Kit?
In the State of Georgia, two kits have been place in the following locations:

All Georgia Learning Resource Centers

The Atlanta Area School for the Deaf

The Georgia School for the Deaf

The Georgia Academy for the Blind

The Georgia P.I.N.E.S. project at AASD

Georgia State University’s teacher preparation program in Deaf Education

The University of Georgia’s teacher preparation program in Deaf Education

Valdosta State University’s teacher preparation program in Deaf Education
For programs outside the state of Georgia, a kit has been given to the regional
Directors for the ACE-DHH catalyst grant. These individuals are:
Sharon Baker- South Central Regional Director
Dee Klein- Northwest Pacific Southwest Regional Director
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Sue Rose- North Central Regional Director
Katharine Slemenda- Southeast Regional Director
Alan Marvelli- Northeast Regional Director
To contact these individuals, go to http://www.deafed.net. Click on “Crossing the
Realities Divide.” This will take you to the ACE-DHH website. Click on “Membership”
and locate the director in your region. In addition, a list of materials and vendors needed
to build your own copy of the kit is available in the handout for this presentation as well
as on the test protocol and in the CD. You may obtain a copy of the test protocol by going
to the Georgia Deafblind Project website (http://education.gsu.edu/georgiadeafblindproj).
In case you forget the address, an easier way to get to the website is to open up a search
engine on the Internet and type in “Georgia Deafblind Project”.
What Does the Screening Kit Evaluate?
This kit evaluates five specific visual skills: visual acuity, field of vision,
dark.light adaptation, balance, and color vision. In addition, a special section for
identifying traits and characteristics associated with Usher Syndrome is included.
Why Is Usher Syndrome Included?
Usher Syndrome is the leading cause of deafblindness acquired after childhood.
The sooner one discovers that a child with a hearing loss has the potential to lose his
vision, the sooner educators can begin to prepare the child to use appropriate adaptations,
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modifications, and available technologies. Usher Syndrome is a constellation of features
that are genetically determined. Individuals with Usher Syndrome have a congenital
hearing loss and a progressive vision loss. An autosomal recessive gene causes Usher
Syndrome. An autosomal recessive gene is a non sex-linked gene, and carriers do not
have the trait themselves. In most cases of Usher Syndrome, the parents do not realize
that they have the gene until their child is diagnosed with the syndrome.
What are the Characteristics of Usher Syndrome?
The three main characteristics of Usher Syndrome are a sensori-neural hearing
loss, retinitis pigmentosa, and the presence of a balance problem. Some cases of Usher
Syndrome present with no balance problem. The characteristics of retinitis pigmentosa
include loss of night visions and reduction in or loss of the visual field.
There are three types of Usher Syndrome. Usher Syndrome Type One is
characterized by a profound hearing loss, balance problems, and retinitis pigmentosa.
Usher Syndrome Type Two is characterized by a congenital hearing loss in the hard-ofhearing range, normal inner ear balance, and retinitis pigmentosa. Individuals with Usher
Syndrome Type Three may be born with a normal to mild hearing loss, have a progressive
loss, may or may not have balance problems, and have retinitis pigmentosa.
The Instructional CD
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The instructional CD that accompanies the handout for this presentation was
designed so that any teacher of the deaf, teacher of the vision impaired, or school nurse
could teacher himself or herself to provide a vision screening to students who are deaf or
hard of hearing. The CD was produced by Susan R. Easterbrooks and Patricia
Montgomery. The CD contains a Powerpoint Presentation of the information presented in
this paper and presented at the conference along with links to pictures and video-clips of
teachers, students, interpreters, parents, and evaluators as they are using the test. As you
watch the presentation on the CD, be sure to click on the links on each slide. The links
are not immediately obvious. You can find a link either by running the cursor over the
slide until the pointing finger icon shows, or you can click the TAB key and it will box
the link. You may open the clip with by hitting ENTER after TAB or by clicking on the
boxed words. This CD requires the Quick Time program in order for the video clips to
run. Special instructions for opening the CD are found on the back of the jewel cover.
The Screening Instrument
There are four parts to the vision screening kit. Part 1 is a form for summarizing
medical information. Part 2 is a questionnaire to be used with the classroom teacher and
the parents. Part 3 is the actual vision screening process itself. Part 4 is the Usher
screening material. Copies of the test protocol are attached provided in the handout
packet.
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Medical Information
It is very important to get as much medical information as possible from both the
teacher and the parents because teachers and parents usually see a child from different
perspectives and in different contexts. The following questions are in the medical profile:
1. What is the cause of the hearing loss?
2. Is there a family history of hearing loss?
3. Are there any known visual problems? (if so, explain)
4. What is the amount and type of hearing loss?
5. Has the student had any eye surgeries? (if so, list)
6. Does the student wear glasses?
7. When was the last vision exam?
8. Does the student have other medical conditions?
The Questionnaire
The questionnaire is filled out by both the parent and the teacher. The person
filling out the questionnaire must circle Y for yes, N for no, and U for unsure regarding
the following statements:
1. Has problems seeing objects far away, but sees well up close. (You will want to
click on Y and N of this slide to see that the teacher and the parents answer this
question differently. This points out how important it is to ask questions of
multiple sources.)
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2. Has problems seeing objects up close, but sees well far away.
3. Holds book or other materials close to eyes or bends to read.
4. Has difficulty seeing at night or in the dark.
5. Has difficulty seeing sign language or gestures in dim light.
6. Has problems reading in dimly lit areas.
7. When entering a new place or going from bright light to dim light (or vice versa)
stops suddenly, stands still, or looks around.
8. Trips over things when light changes or light is dim.
9. Stumbles on stairs and curbs or bumps into things.
10. Fails to glance at another person’s hand waving from the side.
11. Complains of bright light hurting his/her eyes.
12. Squints and shades eyes in bright lights or fluorescent lighting.
13. Likes to wear sunglasses or cap in a building or in bright light.
14. Has difficulty reading light copies of print material.
15. Confuses colors. If yes, the color problem is with: red/green, yellow/blue, or dark
colors.
16. Was late learning to walk (past 15 months).
17. Loses balance easily in the dark.
18. Is considered clumsy.
19. Cannot ride a bicycle or required a long time to learn.
20. Complains of headaches, blurring, itching, rubs eyes, squints, blinks, tilts head,
sees double. Explain.
21. Has one eye or both eyes that turn out.
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22. Has other noticeable vision or balance problems. Explain.
The Vision Screening
Step 1. Eye appearance. Look closely at each eye in good light. Determine if the
general appearance of the eye is normal. If no, indicate on the screening protocol if eyes
are bloodshot, watery, have excessive tearing discharge, have jerky movements, if the
child blinks excessively, or if there are any other noticeable features. Determine if the lids
appear normal. If no, indicate on the protocol if lids are swollen, reddened, drooping,
crusted, or if there are any other noticeable features. Determine if the eyes appear equal
and round. If no, indicate on the protocol if the pupils are unequal, keyhole-shaped, or
otherwise not round. Indicate any other noticeable feature as well.
Step 2. Light reflex. Shine a penlight from below right eye into pupil. Observe if
the right pupil contracts (direct reaction) and left pupil contracts (consensual reaction).
Both eyes should contract. Repeat with the other eye. Move the light from the right eye to
the left eye, noting if both eyes react equally. You may have difficulty noticing a light
reflex in students with dark eyes. In this case, go to a dark or dimly lit room. Wait until
the child’s eyes dark adapt, then shine the light. If you do not see contracting pupils, refer
the child immediately to an ophthalmologist. Non-contracting eyes may be an indicator
of neurological problems or of problems in the functioning of a shunt.
Step 3. Eye position/alignment. Position the student with the light sources behind
him/her (not shining into child’s face). The examiner should position his/her face level
with the student’s face. Hold a well focused pen light 18 inches from the student midway
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between the two eyes (perpendicular to the eye). Tell the student to look at the light.
Observe the position of the light. If you have difficulty seeing the light reflection,
perform this test in a dim room.
Step 4. Fixation. Present an interesting, high contrast, noiseless item (e.g., colorful
finger puppet, colorful toy, light with colored top) approximately 18 inches from the
student. Observe if the student can maintain a steady gaze on the item without eyes
jerking for 3 to 5 seconds. Try this with one eye at a time and both eyes together.
Step 5. Tracking. With the examiner sitting in front of the student, ask him/her to
follow a bright, interesting item with his/her eyes. Start with item at midline and at eye
level. Move item in the following direction: 1) horizontal direction, 2) vertical direction,
3) diagonal direction, 4) circular direction.
Step 6. Peripheral vision. With one person sitting in front of the student to distract
him/her, the other examiner takes a brightly colored, desirable item and slowly brings ti
outward from behind the student at a distance of 12 to 18 inches in an outward arc from
the student. Bring the item out from left, right, top, and bottom periphery.
Step 7. Acuity for distance vision. Use a vision chart for this task. Determine the
appropriate chart prior to beginning the test. Be certain that the student knows the
symbols on the chart. Have an interpreter with you if you are not a skilled signer. Be
aware that colds can affect the results. Do not cue the child. A brightly colored marker
will help the student focus on the symbol. Allow nonverbal student to match symbols.
Alternate direction of reading symbols. The criteria for passing this test is 3/5 symbols in
10/16 line or 4/5 symbols in 10/16 line.
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Step 8. Acuity for near vision. Using the appropriate cards identified in the list at
the end of the protocol, hold the care 16 inches from the student’s eyes. Be careful not to
block light or the student’s visual access. The passing criterion for this test is 3/5 on
20/32 line. Observe and not if the student exhibits the following: thrusting head, tilting
head, watering eyes, squinting, puckering face, excessive blinking, eye pain, frowning.
Step 9. Color testing. Present the student with a pile of paint strips, blocks, or
other items. You will need to have red, green, yellow, blue, brown, and black items. Ask
the student to sort the different colors into piles. As an alternative to this test, you might
use the Ishihara test, or the Good-Lite Pseudo Isochromatic Color Vision Tests. Follow
the specific instructions enclosed with the testing materials.
Usher Screening
In addition to the nine steps above, you will do two additional steps to complete a
screening for Usher Syndrome.
Step 10. Dark/light adaptation (chip sorting). Place white, read, and blue chips
from the Cone Adaptation Test in a pile in a darkened room. Ask the child to pick up the
white chips. Ask the child to sort the red chips from the blue chips. Check for difficulty
and length of time required to complete the task. Next, in a darkened room, conduct a
conversation using sign language (if the student is a sign user) and note if the student
misinterprets any signs. Use signs that are similar in configuration and motion. Finally,
have the student move from outside (or a brightly lit room) into a dimly lit room. Notice
any difficulties in adaptation.
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Step 11. Balance. Have the student place heel of one foot in front of the toes of
the other foot with arms outstretched and eyes closed. If the student is very athletic, you
may gently push the student to see if balance is maintain. Always have another person
ready to catch the student if needed.
What Do I Do With the Vision Screening Results?
If a student fails any part of the Acuity section of the Light/dark Adaptation
section of the vision screening, always refer him/her to an ophthalmologist. In addition,
you will refer a student to the ophthalmologist if he fails the balance part of the screener
AND has: no mental retardation, no multiple system involvement (i.e., neurological),
prelingual deafness, or no history of hearing loss in the family.
Frequently Asked Questions
This next section provides an answer to questions that teachers frequently asked
during the development of the vision screener. These are included to assist new users in
better understanding the application and use of this tool.
1. What are the characteristics of night vision loss?

Difficulty seeing at night or in dimly lit areas.

The student may demonstrate problems walking in familiar environments in dim
light.
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
Parents may notice their child having difficulty walking around at home in
the evening hours.

Young children may consistently want to hold an adult hand at night when
walking in familiar and unfamiliar environments such as the car to home.

Young children may have a consistent fear of the dark and request to sleep
with the lights on. This fear may be due to their inability to see.

Young children may be afraid to go to the bathroom at night because they
can not see in the dark.

Students may demonstrate difficulty adjusting to changes in lighting upon
entering and leaving a building on a bright sunny day by stopping and
standing still allowing time for their eyes to adjust to the change in
illumination.

Some individuals show great difficulty in adjusting to light changes in
movie theaters and demonstrate the same hesitant behavior or avoid movie
theaters altogether.

May demonstrate difficulty with communication in dimly lit areas due to an
inability to read lips or discriminate signs similar in configuration
2. What are the characteristics of a field loss?

The student may have difficulty detecting objects above, below and to the sides

The student may consistently stumble over objects on the floor

The student may bump into walls or desk

The student may not notice a friend waving to him/her from the side
3. What are the characteristics of difficulty with balance?THE CHARACTERISTICS OF
DIFFICULTY WITH BALANCE?
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
Difficulty maintaining or regaining balance particular in the dark

May appear awkward

May appear clumsy
4. What is the retina?
The retina is light sensitive organ in your eye that contains rods and cones. Rods
allow you to see light. Rods also allow you to see on the sides or in the peripheral field.
Cones allow you to see and discriminate color. Cones are also associated with visual
acuity (seeing detail of objects).
5. What is retinitis pigmentosa?
Retinitis Pigmentosa is a progressive degenerative condition of the retina
occurring in both eyes. A person with Retinitis Pigmentosa (RP) typically loses the
ability to see at night followed by gradual loss of peripheral vision (the ability to detect
objects or movement on the sides). Other symptoms include blind spots, which typically
increase in size over many years.
6. How long does it take for a person to lose his or her vision?
There is no way to determine how long it takes for a person with RP to lose
vision. Some lose vision in early childhood and others maintain functional vision into
late adulthood.
7. If a person has a hearing loss and retinitis pigmentosa, does that mean he has Usher
Syndrome?
No. RP (Retinitis Pigmentosa) is also associated with conditions other than Usher
Syndrome.
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8. Who diagnoses Usher Syndrome?
A medical team makes the diagnosis.
9. Where is the best place to position the eye chart during the acuity test?
The eye chart should always be placed at the eye level of the student being tested.
10. What accommodations do I make for children in wheelchairs?
Place the chart at the student’s eye level and position the student’s wheelchair so
that the student’s bottom is at the ten-foot distance mark. Follow the protocol’s directions
for acuity testing.
11.. Does it really matter which eye is tested first?
No. However, it is usually it best to test both eyes first and then test each eye
separately.
12. If the student fails to pass one eye during the test, do I have to keep testing?
No. You may stop that portion of the acuity test but you must continue the
screening with the next section.
13. Do I have the student wear his/her glasses when testing acuity?
If a student wear corrective lenses, ALWAYS test with the glasses on.
14. What if the student is not able occlude his/her eye?
If the student’s is not able to occlude his/her own eye, it is acceptable for someone
to do it for him/her or provide assistance.
15. How do I measure the appropriate distance for the near vision chart.
The black cord attached to the near vision chart is 16 inches in length. Measure
the distance from the eye.
16. What if the student is not able to respond verbally?
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The student may complete the test by matching, pointing or eye gazing using
plastic individual symbol cards.
17. What if the student cannot verbally label the symbols correctly?
Here are several strategies, which may be used.
1. The student may complete the test using a “nonverbal” method as describe above.
2. The student may need prior training. Provide the teacher with a set of symbols
and have the student practice labeling.
3. Before testing, ask the student what they think the symbol should be called.
4. It may help to provide a common association such as a “house---door”, “ball--apple” rather than “house—square”, “circle---heart”.
5. It doesn’t matter what the symbol is called as long as it is “consistent”.
18. I cannot see the student’s eyes dilate or constrict? What do I do?
Sometimes it is difficult to see constriction and dilation on dark eyes. Try the
following suggestions.
1. Be certain the student wasn’t facing a light sources such as the window.
2. Move the student to very dimly lit room or close the blinds.
3. Give enough time for the student’s eye to dilate.
4. Try the procedure again. If you have tried this procedure in a very dark
environment and still do not observe pupil dilation and constriction, contact the
nurse, IMMEDIATELY.
Hints
1. Tilt all blinds upward or completely close them.
2. Be certain the light source comes from behind the student.
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3. Sometimes overhead lights reflect in the student’s eye. Turn the overhead
lights off.
4. Bright light can be painful to some students. Introduce the light slowing from
either below the eye or the outer periphery of the eye. Then gradually guide
the light into position.
5. Complete the procedure as quickly as possible.
6. Try covering the tip of the light with a piece of colored cellophane such as
light red or blue.
7. Be aware of students who are seizure prone. Flashing light can trigger
seizures.
19. What is visual field?
Visual field is the entire area in which you see objects and motion. Visual field
includes the area straight in front of you as well as to your sides.
20. I am not sure how to position the student to test visual field. Do you have any ideas?
x
x
You can mark the general visual field area with masking tape. Set a chair in the
circle position. Using masking tape, make square 18 inches from the chair. Then place
guide marks in the 90-degree and 45-degree positions. This will give an approximation
of the visual field to test.
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180 Degrees
45
45
90
21. What sorts of objects are appropriate for testing visual field?
Any brightly colored object that is non-reflective will work. Brightly color paint
sticks, markers, small cars, finger puppets all suggestions. Be certain that the item is age
appropriate. A toy car may not be the best choice for a sixteen-year-old young man.
22. Do I only test the vision on the sides (peripheral field)?
No. Be sure to test for the awareness of the item from above and below. Loss of
visual field can be center as well as to the side. HINT: Remind the student to keep his/her
head still when doing this test. The student should focus his attention on the person sitting
in front of him/her. This screening requires two (2) people.
23. How will I know if the student sees the object?
Older kids will give you a “thumbs up” signs. Younger kids may respond
verbally with “I see it “ or they may point.
24. How do I test the nonverbal student? How will I know if they see the object?
The assistant should pay close attention to the student’s eye contact. Most
children will turn or make eye contact as soon as an object enters their visual field.
25. My school doesn’t have a room with a rheostat to control the light level. What do I
do?
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You will need to find a very dark place such as an audio-visual room. The lighting
level must able to be lowered to almost complete darkness. Most school libraries have a
room that can be darkened. You can control lightening levels by positioning the door to
the room. (Hint: Be certain the student understands what is going to take place before
beginning. It may be wise to talk with your principal about the purpose of this test and the
procedure prior to administration.)
26. What kind of behaviors do I look for when moving a student from outside (or
brightly-lit room) into a dimly lit room?
Look for excessive reaction to the light. Look for loss of balance. Look for
disorientation. Look for hesitancy in stepping into the room.
27. Why must I have a second person available during the balance section of this
test?
It is critical to have a person standing close to the student being tested in case
he/she falls. Student’s with Usher 1 and possibly Usher 3 are not be able to regain their
balance after being pushed and can fall.
28. Does the student have to close his eye during this portion of the test?
Yes.
Hint: Be sure to stabilize the student before beginning this procedure.
29. My student uses visual sign to communicate and I don’t know sign. What should I
do?
You MUST have an interpreter present during the entire test. This is the only way
to insure reliable results and be fair to the student.
30. My student speaks Spanish and is deaf? Any suggestions?
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Yes. Try to have an ESL (English as a Second Language) teacher available or an
interpreter who speaks your student’s native language. You may be surprised with the
results?
31. What if the family of my student doesn’t speak English? How will I get accurate
answers on the questionnaire section of the screening?
Try the school social worker for suggestions. Most schools will be able to direct
you to the appropriate person. Remember that you may need to ask probing questions.
Sometime the language barriers prevent us from getting important information. An
interpreter will make everyone feel at ease.
32. Do we have to get permission to do this test?
You should follow the guidelines of your school system.
33. What if a student fails the test?
1. Student’s who fail any section of this screening should be referred to an eye
doctor according to the directions in the protocol.
2. Parents should be notified that the student has failed the school vision screening.
3. Each school is encouraged to develop a system of tracking the referral process to
insure that students receive appropriate medical follow-up.
4. It is the position statement of our project that student’s who demonstrate vision
and hearing loss be referred to a pediatric ophthalmologist for evaluation.
Georgia Deafblind Project, Georgia State University, Dept of EPSE, University Plaza, Atlanta, GA 303033083, 404-651-1262 (V/TTY), 404-651-4901 (fax)